Health-related quality of life in children with neurofibromatosis type 1: contribution of demographic factors, disease-related factors, and behavior.
OBJECTIVE
To investigate health-related quality of life (HR-QOL) in children with neurofibromatosis type 1 (NF1) with parental reports and children's self-reports, and to investigate the potential contribution of demographic factors, disease-specific factors, and problems in school performance or behavior.


METHODS
In a prospective observational study, parents of 58 children with NF1 (32 boys, 26 girls, age 12.2 +/- 2.5 years) visiting a university clinic, and their 43 children 10 years or older were assessed with the Child Health Questionnaire (CHQ). Potential determinants of domain scores were assessed in 3 explorative regression models.


RESULTS
Parents reported a significant impact of NF1 on 9/13 CHQ scales, with moderate effect sizes on 8 (general health perceptions, physical functioning, general behavior, mental health, self esteem, family activities, role functioning emotional/behavioral, and parent emotional impact). Children report an impact on bodily pain, and an above average general behavior. Multiple CHQ scales were sensitive to demographic factors and behavioral problems, and 1 to NF1 severity. NF1 visibility and school problems did not influence HR-QOL.


CONCLUSIONS
Parents, but not the children with NF1, report a profound impact of NF1 on physical, social, behavioral, and emotional aspects of HR-QOL. Multiple HR-QOL domains were most sensitive to behavioral problems, which points to an exciting potential opportunity to improve HR-QOL in children with NF1 by addressing these behavioral problems.
Krab
Lianne C
LC
NF1 CoRe Team (Cognitive Research Team), Department of General Pediatrics, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
Oostenbrink
R
R
de Goede-Bolder
Arja
A
Aarsen
Femke K
FK
Elgersma
Ype
Y
Moll
Henriëtte A
HA
D000124:Achievement; D000223:Adaptation, Psychological; D000293:Adolescent; D001294:Attitude to Health; D002648:Child; D002653:Child Behavior Disorders / Q000150:complications; D002669:Child Welfare; D003710:Demography; D005260:Female; D006304:Health Status; D006306:Health Surveys; D006801:Humans; D008297:Male; D008603:Mental Health; D009456:Neurofibromatosis 1 / Q000150:complications / Q000523:psychology*; D010146:Pain / Q000150:complications / Q000523:psychology; D010287:Parent-Child Relations; D010290:Parents; D011446:Prospective Studies; D011788:Quality of Life; D012649:Self Concept; D013315:Stress, Psychological / Q000209:etiology; D011795:Surveys and Questionnaires